The Mitochondrial Lon Protease: Novel Functions off the Beaten Track?

Diverse Cellular Roles of Autophagy

(2019) Hideaki Morishita et al.   Annual Review of Cell and Developmental Biology

Improved mitochondrial stress response in long‐lived Snell dwarf mice

(2019) Ulas Ozkurede et al.   AGING CELL

Global Proteome of LonP1+/− Mouse Embryonal Fibroblasts Reveals Impact on Respiratory Chain, but No Interdependence between Eral1 and Mitoribosomes

(2019) Jana Key et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Analysis of heat-induced protein aggregation in human mitochondria

(2018) Anne Wilkening et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

IMiQ: a novel protein quality control compartment protecting mitochondrial functional integrity

(2018) Michael Bruderek et al.   MOLECULAR BIOLOGY OF THE CELL

LonP1 Differently Modulates Mitochondrial Function and Bioenergetics of Primary Versus Metastatic Colon Cancer Cells

(2018) Lara Gibellini et al.   Frontiers in Oncology

LONP1 Is Required for Maturation of a Subset of Mitochondrial Proteins, and Its Loss Elicits an Integrated Stress Response

(2018) Olga Zurita Rendón et al.   MOLECULAR AND CELLULAR BIOLOGY

Cytosolic proteostasis through importing of misfolded proteins into mitochondria

(2017) Linhao Ruan et al.   NATURE

The coming of age of the mitochondria–ER contact: a matter of thickness

(2016) M Giacomello et al.   CELL DEATH AND DIFFERENTIATION

The integrated stress response

(2016) Karolina Pakos‐Zebrucka et al.   EMBO REPORTS

Protein quality control at the mitochondrion

(2016) W. Voos et al.   Essays in Biochemistry

Protection of scaffold protein Isu from degradation by the Lon protease Pim1 as a component of Fe–S cluster biogenesis regulation

(2016) Szymon J. Ciesielski et al.   MOLECULAR BIOLOGY OF THE CELL

The ever-expanding role of HIF in tumour and stromal biology

(2016) Edward L. LaGory et al.   NATURE CELL BIOLOGY

In vivo aspects of protein folding and quality control

(2016) D. Balchin et al.   SCIENCE

A LON-ClpP Proteolytic Axis Degrades Complex I to Extinguish ROS Production in Depolarized Mitochondria

(2016) Kenneth Robert Pryde et al.   Cell Reports

CODAS Syndrome Is Associated with Mutations of LONP1, Encoding Mitochondrial AAA+ Lon Protease

(2015) Kevin A. Strauss et al.   AMERICAN JOURNAL OF HUMAN GENETICS

The Biology of Proteostasis in Aging and Disease

(2015) Johnathan Labbadia et al.   Annual Review of Biochemistry

Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer

(2015) Marcello Pinti et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Mitochondria Retrograde Signaling and the UPRmt: Where Are We in Mammals?

(2015) Thierry Arnould et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mitochondrial Lon protease controls ROS-dependent apoptosis in cardiomyocyte under hypoxia

(2015) Chan-Yen Kuo et al.   MITOCHONDRION

Transcriptional activation of LON Gene by a new form of mitochondrial stress: A role for the nuclear respiratory factor 2 in StAR overload response (SOR)

(2015) Assaf Bahat et al.   MOLECULAR AND CELLULAR ENDOCRINOLOGY

New roles for mitochondrial proteases in health, ageing and disease

(2015) Pedro M. Quirós et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Structure and function of ER membrane contact sites with other organelles

(2015) Melissa J. Phillips et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mitochondrial Lon regulates apoptosis through the association with Hsp60–mtHsp70 complex

(2015) T-Y Kao et al.   Cell Death & Disease

Tetracyclines Disturb Mitochondrial Function across Eukaryotic Models: A Call for Caution in Biomedical Research

(2015) Norman Moullan et al.   Cell Reports

Inhibition of Lon blocks cell proliferation, enhances chemosensitivity by promoting apoptosis and decreases cellular bioenergetics of bladder cancer: potential roles of Lon as a prognostic marker and therapeutic target in baldder cancer

(2015) Yongzhang Liu et al.   Oncotarget

LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins

(2015) Ayenachew Bezawork-Geleta et al.   Scientific Reports

Silencing of mitochondrial Lon protease deeply impairs mitochondrial proteome and function in colon cancer cells

(2014) Lara Gibellini et al.   FASEB JOURNAL

ATP-Dependent Lon Protease Controls Tumor Bioenergetics by Reprogramming Mitochondrial Activity

(2014) Pedro M. Quirós et al.   Cell Reports

Chaperone machines for protein folding, unfolding and disaggregation

(2013) Helen Saibil   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Overexpression of Lon contributes to survival and aggressive phenotype of cancer cells through mitochondrial complex I-mediated generation of reactive oxygen species

(2013) C-W Cheng et al.   Cell Death & Disease

Upregulation of the mitochondrial Lon Protease allows adaptation to acute oxidative stress but dysregulation is associated with chronic stress, disease, and aging

(2013) Jenny K. Ngo et al.   Redox Biology

Chaperone–protease networks in mitochondrial protein homeostasis

(2012) Wolfgang Voos   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Deletion of the mitochondrial Pim1/Lon protease in yeast results in accelerated aging and impairment of the proteasome

(2012) Nika Erjavec et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial Protein Synthesis, Import, and Assembly

(2012) Thomas D. Fox   GENETICS

Phosphorylation of Human TFAM in Mitochondria Impairs DNA Binding and Promotes Degradation by the AAA+ Lon Protease

(2012) Bin Lu et al.   MOLECULAR CELL

Cysteine desulfurase Nfs1 and Pim1 protease control levels of Isu, the Fe-S cluster biogenesis scaffold

(2012) J.-Y. Song et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

AAA+ Proteases: ATP-Fueled Machines of Protein Destruction

(2011) Robert T. Sauer et al.   Annual Review of Biochemistry

Multitasking in the mitochondrion by the ATP-dependent Lon protease

(2011) Sundararajan Venkatesh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Impairment of Lon-Induced Protection Against the Accumulation of Oxidized Proteins in Senescent Wi-38 Fibroblasts

(2011) J. K. Ngo et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

Mitochondrial enzymes are protected from stress-induced aggregation by mitochondrial chaperones and the Pim1/LON protease

(2011) Tom Bender et al.   MOLECULAR BIOLOGY OF THE CELL

The quantitative proteome of a human cell line

(2011) M. Beck et al.   Molecular Systems Biology

Identification of Novel Oxidized Protein Substrates and Physiological Partners of the Mitochondrial ATP-dependent Lon-like Protease Pim1

(2010) Aurélien Bayot et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM)

(2010) Y. Matsushima et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The role of protein quality control in mitochondrial protein homeostasis under oxidative stress

(2010) Tom Bender et al.   PROTEOMICS

Mitochondrial Lon protease is a human stress protein

(2009) Jenny K. Ngo et al.   FREE RADICAL BIOLOGY AND MEDICINE

Increasing organismal healthspan by enhancing mitochondrial protein quality control

(2009) Karin Luce et al.   NATURE CELL BIOLOGY

Isolation of mitochondria-associated membranes and mitochondria from animal tissues and cells

(2009) Mariusz R Wieckowski et al.   Nature Protocols

Understanding the Warburg Effect: The Metabolic Requirements of Cell Proliferation

(2009) M. G. Vander Heiden et al.   SCIENCE